John E. Schneider, MD

Case

An 8-week-old male infant was brought to the ED by his parents after an episode in which it appeared the baby had stopped breathing. The parents stated that while lying on his mother’s lap at home, the patient stopped breathing for approximately 10 to 15 seconds, during which time his face exhibited a bluish color. They further noted that the patient began breathing again after gentle stimulation and had been acting normally since.

The patient was born at 39 weeks gestation via normal vaginal delivery and without any complications. His parents further stated that prior to the cessation of breathing incident, his symptoms of nasal congestion, decreased energy level, and fast breathing had gradually worsened over the past 2 days. The parents also noted that the infant had not been feeding as well over the past 2 days.

Upon arrival, the patient’s vital signs were: heart rate, 140 beats/minute; respiratory rate (RR), 72 beats/minute; and temperature 101.3°F. Oxygen saturation was 92% on room air. On physical examination, the infant had significant rhinorrhea, moderate intercostal and supraclavicular retractions, ausculatory wheezes, and transmitted upper airway noises throughout.

  

Overview

Bronchiolitis, a disorder caused by a viral lower respiratory tract infection, is the most common lower respiratory infection in children younger than age 2 years.¹ In 2014, the American Academy of Pediatrics (AAP) characterized bronchiolitis as “rhinitis, cough, tachypnea, wheezing, rales, use of accessory muscles, and/or nasal flaring in children under 24 months of age.”² This condition is the most common cause of hospitalization in the first 12 months of life. It is responsible for over 100,000 admissions annually at an estimated cost to the healthcare system of $1.73 billion.³

  

Etiology and Pathophysiology

Respiratory syncytial virus (RSV) is the most common cause of bronchiolitis. In the United States, the highest incidence of infection occurs during the months of December through March, with some degree of regional variability.⁴ A number of other viruses that can cause bronchiolitis include human metapneumovirus, parainfluenza virus, and influenza virus.¹ Infection with RSV does not grant permanent immunity, and reinfection is common throughout life.²

Pathophysiologically, bronchiolitis is characterized by an invasion of bronchial epithelial cells that lead to to cell death and sloughing into the bronchial lumen. This, coupled with increased mucous production and submucosal edema, leads to a narrowing of the bronchial lumen and obstruction of airflow.⁵

  

Clinical Manifestations

Bronchiolitis represents a constellation of signs and symptoms beginning with those of an upper respiratory tract infection, including nasal congestion and rhinorrhea with mild cough. On days 3 to 5, the following symptoms develop: tachypnea, wheezing, rales, and signs of respiratory distress (eg, grunting, nasal flaring, inter-/subcostal retractions). Approximately two-thirds of patients will develop a fever.² Recovery tends to begin around days 5 to 7, with the median duration of illness being 12 days.¹ It should be noted that bronchiolitis represents a highly variable and dynamic disease state. Transient episodes of improvement and worsening are common, emphasizing the importance of serial examinations and assessments. Though rare, progression to respiratory failure and death do occur.²

  

History and Risk Stratification

The focus of the initial history by the clinician should serve two primary purposes. First, it is important to differentiate infants with probable bronchiolitis from those with other disease states having similar clinical manifestations. One of the most challenging diseases to differentiate from bronchiolitis is that of reactive airway disease (RAD). Eliciting a history of allergic rhinitis, eczema, or a family history of asthma may be helpful in determining the precise etiology of the patient’s symptoms. Although no longer recommended for children with bronchiolitis (as will be later discussed), a trial of a bronchodilation may be beneficial in the setting of familial atopy.

The second—and perhaps most important—aspect of patient history is to determine the presence of risk factors for both apnea and the development of severe bronchiolitis. Regarding the risk factors for apnea, Willwerth et al⁶ developed a set of criteria to identify patients at high risk for apnea in the inpatient setting. Patients were considered high risk if they were born at full term and were younger than 1 month of age; if they were born preterm (<37 weeks gestation) and were younger than 48 weeks postconception; and/or if the infant’s parents or a clinician had already witnessed an episode of apnea during the patient’s illness. In this study, all patients who developed apnea were correctly identified by the risk criteria.⁶ Risk factors for severe bronchiolitis include the following: patient age younger than 12 weeks; patient prematurity younger than 37 weeks gestation; and an underlying hemodynamically significant congenital heart disease, chronic lung disease/bronchopulmonary dysplasia, or an immunocompromised state.¹

 

  

Diagnosis

In 2014, the AAP updated its guidelines on the diagnosis, management, and prevention of bronchiolitis. One of the strongest statements in these guidelines emphasize that the diagnosis of bronchiolitis should be based almost exclusively on the history and physical examination.² In children younger than age 2 years, historical features such as a viral prodrome, followed by progressively worsening increased respiratory effort and signs and symptoms of lower respiratory-tract disease (eg, wheezing), should guide clinicians to the diagnosis of bronchiolitis. Although nonspecific, physical examination findings such as rhinorrhea, cough, tachypnea, wheezing, rales, and increased respiratory effort—when coupled with a good history—can be beneficial in the diagnosis of bronchiolitis.

Pulse Oximetry

Pulse oximetry has become a standard part of the clinical assessment of patients with bronchiolitis. This is based on data suggesting that pulse oximetry detects hypoxia in cases where it was not suspected on physical examination alone.⁷ However, the effectiveness of pulse oximetry in predicting clinical outcomes is limited. Pulse oximetry should not be used as a proxy for respiratory distress, as studies have shown poor correlation between respiratory distress and oxygen saturations in infants with lower respiratory tract infection.⁸

Radiographic Evaluation

Regarding the diagnosis of bronchiolitis, the AAP notes, “radiographic and laboratory studies should not be obtained routinely.”² While many children with bronchiolitis may have abnormalities on radiographs, there is insufficient data to suggest that chest radiographs correlate with disease severity. In addition, several studies, including a prospective cohort study by Schuh et al,⁹ have shown that infants with suspected lower respiratory tract infections who undergo radiography are more likely to receive antibiotics without any difference in outcomes.

Laboratory Studies

As stated in the AAP guidelines, routine laboratory testing, particularly virologic studies for RSV, have little role in the diagnosis of bronchiolitis. Since numerous viruses can cause bronchiolitis and have similar clinical presentations, the absence of identification of a particular virologic agent does not exclude the diagnosis of bronchiolitis and is moreover unlikely to alter management.

Although routine laboratory evaluation is not recommended in infants with bronchiolitis, one subgroup in which it may be beneficial is in the assessment of serious bacterial infections (SBIs) in febrile infants with bronchiolitis who are younger than 60 days old. Levine et al¹⁰ conducted a large, multicenter, prospective, cross-sectional study of young, febrile infants to determine the risk of SBI in those with RSV bronchiolitis versus those without RSV bronchiolitis. They found that overall febrile infants younger than age 60 days with RSV bronchiolitis have a lower rate of SBI than those without RSV (7% v 12.5%, respectively).¹⁰ In infants between age 28 and 60 days with RSV bronchiolitis, the origin of all SBIs in the study were urinary tract infections. In patients younger than 28 days of age, the risk of developing an SBI was found to be no different between the RSV-positive and RSV-negative groups.¹⁰

Based on the findings in this study, it is recommended that, at the very least, urinalysis for bacterial infection be performed in all infants with RSV bronchiolitis who are younger than age 60 days. Furthermore, since there was no difference in the rates of SBI in patients younger than age 28 days, infants in this age range should undergo a full septic work-up (blood, urine, and cerebrospinal fluid)—regardless of RSV infection status. For infants between ages 28 and 60 days, there is not enough evidence to recommend for or against further laboratory evaluation other than urinalysis.

  

Treatment

Nasal Suctioning

Nasal suctioning has become the first-line treatment for infants with bronchiolitis. It is used to clear secretions from the nasal passages to aid in respiration, which is particularly important in younger infants—who are obligate nose breathers. Current recommendations are to perform suctioning with increasing respiratory effort, before feeding and before laying the infant down to sleep.¹

Bronchodilators

In the past, bronchodilators such as the β-agonist albuterol have been used to treat bronchiolitis with the idea that bronchial smooth muscle relaxation would improve clinical symptoms. In its 2006 guidelines, the AAP had recommended a trial of albuterol and continuation only if there was a documented objective response. In the 2014 updated guidelines, however, the AAP no longer recommends the use of albuterol in any capacity.

Although several meta-analyses and systematic reviews have demonstrated that bronchodilators may improve clinical symptoms scores, they did not affect disease resolution, need for hospitalization, or length of hospital stay.² In addition, a recent Cochrane systematic review noted no benefit in the clinical course of infants with bronchiolitis treated with bronchodilators, and cited the potential adverse events (tachycardia and tremors) as outweighing any potential benefit.¹¹ In addition to albuterol, the AAP no longer recommends the use of nebulized epinephrine in the treatment of bronchiolitis.²

 

Hypertonic Saline

Although hypertonic saline (HTS) has been increasingly studied for the treatment of bronchiolitis, the AAP does not recommend its use in the ED. Despite evidence that HTS may reduce hospital length of stay after 24 hours of use in settings where the typical duration of hospitalization exceeds 3 days, it has not been shown to reduce the rate of hospitalization when used in an emergency setting.²

Corticosteroids

While there is good evidence that corticosteroids are beneficial in treating some respiratory diseases, such as asthma and croup, numerous studies have repeatedly failed to show a benefit in treating bronchiolitis. One of the largest studies, a multicenter, randomized, controlled trial of dexamethasone for bronchiolitis by the Pediatric Emergency Care Applied Research Network, did not show any alteration in admission rates, respiratory status after 4 hours of observation, or length of hospital stay.¹² Accordingly, the AAP strongly recommends against the administration of corticosteroids for bronchiolitis in any setting.²

Oxygen Therapy

Oxygen therapy is often necessary in patients with bronchiolitis who demonstrate hypoxia. The definition of hypoxia in this patient population has remained variable. The AAP has established a threshold of oxyhemoglobin saturation (SpO₂) of less than 90% to define hypoxia and has empowered clinicians to not administer oxygen if the SpO₂ exceeds 90%. Based on the oxyhemoglobin dissociation curve, the authors of the AAP guidelines note that when the SpO₂ is less than 90%, small decreases in the arterial partial pressure of oxygen (PaO₂) result in large decreases in the SpO₂. When SpO₂ is greater than 90%, however, large increases in PaO₂ are associated with only small increased in SpO₂. The AAP guidelines note, “In infants and children with bronchiolitis, no data exist to suggest that such increases [in PaO₂ and SpO₂] result in any clinically significant differences in physiologic function, patient symptoms, or clinical outcomes.”²

A relatively new method of administration of oxygen to infants with bronchiolitis is via a humidified, heated, high-flow nasal cannula (HHHFNC). This therapy has been shown to generate continuous positive airway pressure, which improves respiratory effort, reduces the work of breathing, and may decrease the need for intubation.²

  

Patient Disposition

One of the most challenging tasks for emergency physicians (EPs) is determining the appropriate disposition of infants with bronchiolitis. The variable presentation and dynamic nature of the disease make this particularly difficult. Patients at high risk for apnea should be admitted to the hospital for observation and further care as needed. Admission also should be strongly considered for those with significantly increased work of breathing and tachypnea that does not improve with suctioning—especially when these interfere with feeding. Infants with poor feeding or evidence of dehydration should be admitted to the hospital for intravenous (IV) fluid hydration or nasogastric feedings. Patients with hypoxia (SpO₂ saturations <90%) should also be admitted for supplemental oxygen therapy. It should be noted, however, the AAP recommends “spot-checks” over continuous pulse oximetry in patients who do not require oxygen therapy.²

Another important factor affecting patient disposition is the ability of the caregiver to provide basic patient care and ensure close outpatient follow-up. Prior to discharge, caregivers should be educated on the highly dynamic nature of bronchiolitis and the signs and symptoms that would require prompt return to the ED—especially if the infant has risk factors for the development of severe disease.

  

Case Conclusion

Based on the patient’s symptoms, history (most notably, the recent incident of sleep apnea at home), and physical examination, the EP quickly identified this infant was at a high risk for both severe bronchiolitis and apnea and required aggressive management. Nasal suctioning was immediately performed to help clear the patient’s secretions; this, however, only slightly improved his RR and work of breathing. Although the infant’s SpO₂ was greater than 90% on room air, the EP administered oxygen via HHHFNC at 6 L per minute, which produced a significant improvement in both RR and effort.

Given the patient’s age and the presence of a fever, a urinalysis was also obtained, the results of which showed no evidence of infection. Since the patient was only able to bottle-feed for a few minutes at a time, the EP initiated IV fluid hydration and contacted the hospitalist team for inpatient admission.

The infant was gradually weaned from HHHFNC on hospital day 2 but remained with suboptimal oral intake for another 24 hours. By hospital day 4, his work of breathing had improved significantly, and he was feeding well with through the assistance of pre-feeding nasal syringe suctioning. The patient was discharged home in the care of his parents later that same day with only mild tachypnea over baseline. At discharge, the EP emphasized the importance of providing close follow-up with their son’s pediatrician. The infant continued to gradually improve as an outpatient, with resolution of nasal congestion by day 12 of his illness; he returned to his baseline breathing and feeding pattern on day 14.

 

  

---

Dr Schneider is a pediatric emergency medicine fellow, Eastern Virginia Medical School, Children’s Hospital of The King’s Daughters, Norfolk. Dr Clingenpeel is a fellowship director of pediatric emergency medicine, and an associate professor of pediatrics, Eastern Virginia Medical School, Norfolk.

Case

An 8-week-old male infant was brought to the ED by his parents after an episode in which it appeared the baby had stopped breathing. The parents stated that while lying on his mother’s lap at home, the patient stopped breathing for approximately 10 to 15 seconds, during which time his face exhibited a bluish color. They further noted that the patient began breathing again after gentle stimulation and had been acting normally since.

The patient was born at 39 weeks gestation via normal vaginal delivery and without any complications. His parents further stated that prior to the cessation of breathing incident, his symptoms of nasal congestion, decreased energy level, and fast breathing had gradually worsened over the past 2 days. The parents also noted that the infant had not been feeding as well over the past 2 days.

Upon arrival, the patient’s vital signs were: heart rate, 140 beats/minute; respiratory rate (RR), 72 beats/minute; and temperature 101.3°F. Oxygen saturation was 92% on room air. On physical examination, the infant had significant rhinorrhea, moderate intercostal and supraclavicular retractions, ausculatory wheezes, and transmitted upper airway noises throughout.

  

Overview

Bronchiolitis, a disorder caused by a viral lower respiratory tract infection, is the most common lower respiratory infection in children younger than age 2 years.¹ In 2014, the American Academy of Pediatrics (AAP) characterized bronchiolitis as “rhinitis, cough, tachypnea, wheezing, rales, use of accessory muscles, and/or nasal flaring in children under 24 months of age.”² This condition is the most common cause of hospitalization in the first 12 months of life. It is responsible for over 100,000 admissions annually at an estimated cost to the healthcare system of $1.73 billion.³

  

Etiology and Pathophysiology

Respiratory syncytial virus (RSV) is the most common cause of bronchiolitis. In the United States, the highest incidence of infection occurs during the months of December through March, with some degree of regional variability.⁴ A number of other viruses that can cause bronchiolitis include human metapneumovirus, parainfluenza virus, and influenza virus.¹ Infection with RSV does not grant permanent immunity, and reinfection is common throughout life.²

Pathophysiologically, bronchiolitis is characterized by an invasion of bronchial epithelial cells that lead to to cell death and sloughing into the bronchial lumen. This, coupled with increased mucous production and submucosal edema, leads to a narrowing of the bronchial lumen and obstruction of airflow.⁵

  

Clinical Manifestations

Bronchiolitis represents a constellation of signs and symptoms beginning with those of an upper respiratory tract infection, including nasal congestion and rhinorrhea with mild cough. On days 3 to 5, the following symptoms develop: tachypnea, wheezing, rales, and signs of respiratory distress (eg, grunting, nasal flaring, inter-/subcostal retractions). Approximately two-thirds of patients will develop a fever.² Recovery tends to begin around days 5 to 7, with the median duration of illness being 12 days.¹ It should be noted that bronchiolitis represents a highly variable and dynamic disease state. Transient episodes of improvement and worsening are common, emphasizing the importance of serial examinations and assessments. Though rare, progression to respiratory failure and death do occur.²

  

History and Risk Stratification

The focus of the initial history by the clinician should serve two primary purposes. First, it is important to differentiate infants with probable bronchiolitis from those with other disease states having similar clinical manifestations. One of the most challenging diseases to differentiate from bronchiolitis is that of reactive airway disease (RAD). Eliciting a history of allergic rhinitis, eczema, or a family history of asthma may be helpful in determining the precise etiology of the patient’s symptoms. Although no longer recommended for children with bronchiolitis (as will be later discussed), a trial of a bronchodilation may be beneficial in the setting of familial atopy.

The second—and perhaps most important—aspect of patient history is to determine the presence of risk factors for both apnea and the development of severe bronchiolitis. Regarding the risk factors for apnea, Willwerth et al⁶ developed a set of criteria to identify patients at high risk for apnea in the inpatient setting. Patients were considered high risk if they were born at full term and were younger than 1 month of age; if they were born preterm (<37 weeks gestation) and were younger than 48 weeks postconception; and/or if the infant’s parents or a clinician had already witnessed an episode of apnea during the patient’s illness. In this study, all patients who developed apnea were correctly identified by the risk criteria.⁶ Risk factors for severe bronchiolitis include the following: patient age younger than 12 weeks; patient prematurity younger than 37 weeks gestation; and an underlying hemodynamically significant congenital heart disease, chronic lung disease/bronchopulmonary dysplasia, or an immunocompromised state.¹

 

  

Diagnosis

In 2014, the AAP updated its guidelines on the diagnosis, management, and prevention of bronchiolitis. One of the strongest statements in these guidelines emphasize that the diagnosis of bronchiolitis should be based almost exclusively on the history and physical examination.² In children younger than age 2 years, historical features such as a viral prodrome, followed by progressively worsening increased respiratory effort and signs and symptoms of lower respiratory-tract disease (eg, wheezing), should guide clinicians to the diagnosis of bronchiolitis. Although nonspecific, physical examination findings such as rhinorrhea, cough, tachypnea, wheezing, rales, and increased respiratory effort—when coupled with a good history—can be beneficial in the diagnosis of bronchiolitis.

Pulse Oximetry

Pulse oximetry has become a standard part of the clinical assessment of patients with bronchiolitis. This is based on data suggesting that pulse oximetry detects hypoxia in cases where it was not suspected on physical examination alone.⁷ However, the effectiveness of pulse oximetry in predicting clinical outcomes is limited. Pulse oximetry should not be used as a proxy for respiratory distress, as studies have shown poor correlation between respiratory distress and oxygen saturations in infants with lower respiratory tract infection.⁸

Radiographic Evaluation

Regarding the diagnosis of bronchiolitis, the AAP notes, “radiographic and laboratory studies should not be obtained routinely.”² While many children with bronchiolitis may have abnormalities on radiographs, there is insufficient data to suggest that chest radiographs correlate with disease severity. In addition, several studies, including a prospective cohort study by Schuh et al,⁹ have shown that infants with suspected lower respiratory tract infections who undergo radiography are more likely to receive antibiotics without any difference in outcomes.

Laboratory Studies

As stated in the AAP guidelines, routine laboratory testing, particularly virologic studies for RSV, have little role in the diagnosis of bronchiolitis. Since numerous viruses can cause bronchiolitis and have similar clinical presentations, the absence of identification of a particular virologic agent does not exclude the diagnosis of bronchiolitis and is moreover unlikely to alter management.

Although routine laboratory evaluation is not recommended in infants with bronchiolitis, one subgroup in which it may be beneficial is in the assessment of serious bacterial infections (SBIs) in febrile infants with bronchiolitis who are younger than 60 days old. Levine et al¹⁰ conducted a large, multicenter, prospective, cross-sectional study of young, febrile infants to determine the risk of SBI in those with RSV bronchiolitis versus those without RSV bronchiolitis. They found that overall febrile infants younger than age 60 days with RSV bronchiolitis have a lower rate of SBI than those without RSV (7% v 12.5%, respectively).¹⁰ In infants between age 28 and 60 days with RSV bronchiolitis, the origin of all SBIs in the study were urinary tract infections. In patients younger than 28 days of age, the risk of developing an SBI was found to be no different between the RSV-positive and RSV-negative groups.¹⁰

Based on the findings in this study, it is recommended that, at the very least, urinalysis for bacterial infection be performed in all infants with RSV bronchiolitis who are younger than age 60 days. Furthermore, since there was no difference in the rates of SBI in patients younger than age 28 days, infants in this age range should undergo a full septic work-up (blood, urine, and cerebrospinal fluid)—regardless of RSV infection status. For infants between ages 28 and 60 days, there is not enough evidence to recommend for or against further laboratory evaluation other than urinalysis.

  

Treatment

Nasal Suctioning

Nasal suctioning has become the first-line treatment for infants with bronchiolitis. It is used to clear secretions from the nasal passages to aid in respiration, which is particularly important in younger infants—who are obligate nose breathers. Current recommendations are to perform suctioning with increasing respiratory effort, before feeding and before laying the infant down to sleep.¹

Bronchodilators

In the past, bronchodilators such as the β-agonist albuterol have been used to treat bronchiolitis with the idea that bronchial smooth muscle relaxation would improve clinical symptoms. In its 2006 guidelines, the AAP had recommended a trial of albuterol and continuation only if there was a documented objective response. In the 2014 updated guidelines, however, the AAP no longer recommends the use of albuterol in any capacity.

Although several meta-analyses and systematic reviews have demonstrated that bronchodilators may improve clinical symptoms scores, they did not affect disease resolution, need for hospitalization, or length of hospital stay.² In addition, a recent Cochrane systematic review noted no benefit in the clinical course of infants with bronchiolitis treated with bronchodilators, and cited the potential adverse events (tachycardia and tremors) as outweighing any potential benefit.¹¹ In addition to albuterol, the AAP no longer recommends the use of nebulized epinephrine in the treatment of bronchiolitis.²

 

Hypertonic Saline

Although hypertonic saline (HTS) has been increasingly studied for the treatment of bronchiolitis, the AAP does not recommend its use in the ED. Despite evidence that HTS may reduce hospital length of stay after 24 hours of use in settings where the typical duration of hospitalization exceeds 3 days, it has not been shown to reduce the rate of hospitalization when used in an emergency setting.²

Corticosteroids

While there is good evidence that corticosteroids are beneficial in treating some respiratory diseases, such as asthma and croup, numerous studies have repeatedly failed to show a benefit in treating bronchiolitis. One of the largest studies, a multicenter, randomized, controlled trial of dexamethasone for bronchiolitis by the Pediatric Emergency Care Applied Research Network, did not show any alteration in admission rates, respiratory status after 4 hours of observation, or length of hospital stay.¹² Accordingly, the AAP strongly recommends against the administration of corticosteroids for bronchiolitis in any setting.²

Oxygen Therapy

Oxygen therapy is often necessary in patients with bronchiolitis who demonstrate hypoxia. The definition of hypoxia in this patient population has remained variable. The AAP has established a threshold of oxyhemoglobin saturation (SpO₂) of less than 90% to define hypoxia and has empowered clinicians to not administer oxygen if the SpO₂ exceeds 90%. Based on the oxyhemoglobin dissociation curve, the authors of the AAP guidelines note that when the SpO₂ is less than 90%, small decreases in the arterial partial pressure of oxygen (PaO₂) result in large decreases in the SpO₂. When SpO₂ is greater than 90%, however, large increases in PaO₂ are associated with only small increased in SpO₂. The AAP guidelines note, “In infants and children with bronchiolitis, no data exist to suggest that such increases [in PaO₂ and SpO₂] result in any clinically significant differences in physiologic function, patient symptoms, or clinical outcomes.”²

A relatively new method of administration of oxygen to infants with bronchiolitis is via a humidified, heated, high-flow nasal cannula (HHHFNC). This therapy has been shown to generate continuous positive airway pressure, which improves respiratory effort, reduces the work of breathing, and may decrease the need for intubation.²

  

Patient Disposition

One of the most challenging tasks for emergency physicians (EPs) is determining the appropriate disposition of infants with bronchiolitis. The variable presentation and dynamic nature of the disease make this particularly difficult. Patients at high risk for apnea should be admitted to the hospital for observation and further care as needed. Admission also should be strongly considered for those with significantly increased work of breathing and tachypnea that does not improve with suctioning—especially when these interfere with feeding. Infants with poor feeding or evidence of dehydration should be admitted to the hospital for intravenous (IV) fluid hydration or nasogastric feedings. Patients with hypoxia (SpO₂ saturations <90%) should also be admitted for supplemental oxygen therapy. It should be noted, however, the AAP recommends “spot-checks” over continuous pulse oximetry in patients who do not require oxygen therapy.²

Another important factor affecting patient disposition is the ability of the caregiver to provide basic patient care and ensure close outpatient follow-up. Prior to discharge, caregivers should be educated on the highly dynamic nature of bronchiolitis and the signs and symptoms that would require prompt return to the ED—especially if the infant has risk factors for the development of severe disease.

  

Case Conclusion

Based on the patient’s symptoms, history (most notably, the recent incident of sleep apnea at home), and physical examination, the EP quickly identified this infant was at a high risk for both severe bronchiolitis and apnea and required aggressive management. Nasal suctioning was immediately performed to help clear the patient’s secretions; this, however, only slightly improved his RR and work of breathing. Although the infant’s SpO₂ was greater than 90% on room air, the EP administered oxygen via HHHFNC at 6 L per minute, which produced a significant improvement in both RR and effort.

Given the patient’s age and the presence of a fever, a urinalysis was also obtained, the results of which showed no evidence of infection. Since the patient was only able to bottle-feed for a few minutes at a time, the EP initiated IV fluid hydration and contacted the hospitalist team for inpatient admission.

The infant was gradually weaned from HHHFNC on hospital day 2 but remained with suboptimal oral intake for another 24 hours. By hospital day 4, his work of breathing had improved significantly, and he was feeding well with through the assistance of pre-feeding nasal syringe suctioning. The patient was discharged home in the care of his parents later that same day with only mild tachypnea over baseline. At discharge, the EP emphasized the importance of providing close follow-up with their son’s pediatrician. The infant continued to gradually improve as an outpatient, with resolution of nasal congestion by day 12 of his illness; he returned to his baseline breathing and feeding pattern on day 14.

 

  

---

Dr Schneider is a pediatric emergency medicine fellow, Eastern Virginia Medical School, Children’s Hospital of The King’s Daughters, Norfolk. Dr Clingenpeel is a fellowship director of pediatric emergency medicine, and an associate professor of pediatrics, Eastern Virginia Medical School, Norfolk.

Case

An 8-week-old male infant was brought to the ED by his parents after an episode in which it appeared the baby had stopped breathing. The parents stated that while lying on his mother’s lap at home, the patient stopped breathing for approximately 10 to 15 seconds, during which time his face exhibited a bluish color. They further noted that the patient began breathing again after gentle stimulation and had been acting normally since.

The patient was born at 39 weeks gestation via normal vaginal delivery and without any complications. His parents further stated that prior to the cessation of breathing incident, his symptoms of nasal congestion, decreased energy level, and fast breathing had gradually worsened over the past 2 days. The parents also noted that the infant had not been feeding as well over the past 2 days.

Upon arrival, the patient’s vital signs were: heart rate, 140 beats/minute; respiratory rate (RR), 72 beats/minute; and temperature 101.3°F. Oxygen saturation was 92% on room air. On physical examination, the infant had significant rhinorrhea, moderate intercostal and supraclavicular retractions, ausculatory wheezes, and transmitted upper airway noises throughout.

  

Overview

Bronchiolitis, a disorder caused by a viral lower respiratory tract infection, is the most common lower respiratory infection in children younger than age 2 years.¹ In 2014, the American Academy of Pediatrics (AAP) characterized bronchiolitis as “rhinitis, cough, tachypnea, wheezing, rales, use of accessory muscles, and/or nasal flaring in children under 24 months of age.”² This condition is the most common cause of hospitalization in the first 12 months of life. It is responsible for over 100,000 admissions annually at an estimated cost to the healthcare system of $1.73 billion.³

  

Etiology and Pathophysiology

Respiratory syncytial virus (RSV) is the most common cause of bronchiolitis. In the United States, the highest incidence of infection occurs during the months of December through March, with some degree of regional variability.⁴ A number of other viruses that can cause bronchiolitis include human metapneumovirus, parainfluenza virus, and influenza virus.¹ Infection with RSV does not grant permanent immunity, and reinfection is common throughout life.²

Pathophysiologically, bronchiolitis is characterized by an invasion of bronchial epithelial cells that lead to to cell death and sloughing into the bronchial lumen. This, coupled with increased mucous production and submucosal edema, leads to a narrowing of the bronchial lumen and obstruction of airflow.⁵

  

Clinical Manifestations

Bronchiolitis represents a constellation of signs and symptoms beginning with those of an upper respiratory tract infection, including nasal congestion and rhinorrhea with mild cough. On days 3 to 5, the following symptoms develop: tachypnea, wheezing, rales, and signs of respiratory distress (eg, grunting, nasal flaring, inter-/subcostal retractions). Approximately two-thirds of patients will develop a fever.² Recovery tends to begin around days 5 to 7, with the median duration of illness being 12 days.¹ It should be noted that bronchiolitis represents a highly variable and dynamic disease state. Transient episodes of improvement and worsening are common, emphasizing the importance of serial examinations and assessments. Though rare, progression to respiratory failure and death do occur.²

  

History and Risk Stratification

The focus of the initial history by the clinician should serve two primary purposes. First, it is important to differentiate infants with probable bronchiolitis from those with other disease states having similar clinical manifestations. One of the most challenging diseases to differentiate from bronchiolitis is that of reactive airway disease (RAD). Eliciting a history of allergic rhinitis, eczema, or a family history of asthma may be helpful in determining the precise etiology of the patient’s symptoms. Although no longer recommended for children with bronchiolitis (as will be later discussed), a trial of a bronchodilation may be beneficial in the setting of familial atopy.

The second—and perhaps most important—aspect of patient history is to determine the presence of risk factors for both apnea and the development of severe bronchiolitis. Regarding the risk factors for apnea, Willwerth et al⁶ developed a set of criteria to identify patients at high risk for apnea in the inpatient setting. Patients were considered high risk if they were born at full term and were younger than 1 month of age; if they were born preterm (<37 weeks gestation) and were younger than 48 weeks postconception; and/or if the infant’s parents or a clinician had already witnessed an episode of apnea during the patient’s illness. In this study, all patients who developed apnea were correctly identified by the risk criteria.⁶ Risk factors for severe bronchiolitis include the following: patient age younger than 12 weeks; patient prematurity younger than 37 weeks gestation; and an underlying hemodynamically significant congenital heart disease, chronic lung disease/bronchopulmonary dysplasia, or an immunocompromised state.¹

 

  

Diagnosis

In 2014, the AAP updated its guidelines on the diagnosis, management, and prevention of bronchiolitis. One of the strongest statements in these guidelines emphasize that the diagnosis of bronchiolitis should be based almost exclusively on the history and physical examination.² In children younger than age 2 years, historical features such as a viral prodrome, followed by progressively worsening increased respiratory effort and signs and symptoms of lower respiratory-tract disease (eg, wheezing), should guide clinicians to the diagnosis of bronchiolitis. Although nonspecific, physical examination findings such as rhinorrhea, cough, tachypnea, wheezing, rales, and increased respiratory effort—when coupled with a good history—can be beneficial in the diagnosis of bronchiolitis.

Pulse Oximetry

Pulse oximetry has become a standard part of the clinical assessment of patients with bronchiolitis. This is based on data suggesting that pulse oximetry detects hypoxia in cases where it was not suspected on physical examination alone.⁷ However, the effectiveness of pulse oximetry in predicting clinical outcomes is limited. Pulse oximetry should not be used as a proxy for respiratory distress, as studies have shown poor correlation between respiratory distress and oxygen saturations in infants with lower respiratory tract infection.⁸

Radiographic Evaluation

Regarding the diagnosis of bronchiolitis, the AAP notes, “radiographic and laboratory studies should not be obtained routinely.”² While many children with bronchiolitis may have abnormalities on radiographs, there is insufficient data to suggest that chest radiographs correlate with disease severity. In addition, several studies, including a prospective cohort study by Schuh et al,⁹ have shown that infants with suspected lower respiratory tract infections who undergo radiography are more likely to receive antibiotics without any difference in outcomes.

Laboratory Studies

As stated in the AAP guidelines, routine laboratory testing, particularly virologic studies for RSV, have little role in the diagnosis of bronchiolitis. Since numerous viruses can cause bronchiolitis and have similar clinical presentations, the absence of identification of a particular virologic agent does not exclude the diagnosis of bronchiolitis and is moreover unlikely to alter management.

Although routine laboratory evaluation is not recommended in infants with bronchiolitis, one subgroup in which it may be beneficial is in the assessment of serious bacterial infections (SBIs) in febrile infants with bronchiolitis who are younger than 60 days old. Levine et al¹⁰ conducted a large, multicenter, prospective, cross-sectional study of young, febrile infants to determine the risk of SBI in those with RSV bronchiolitis versus those without RSV bronchiolitis. They found that overall febrile infants younger than age 60 days with RSV bronchiolitis have a lower rate of SBI than those without RSV (7% v 12.5%, respectively).¹⁰ In infants between age 28 and 60 days with RSV bronchiolitis, the origin of all SBIs in the study were urinary tract infections. In patients younger than 28 days of age, the risk of developing an SBI was found to be no different between the RSV-positive and RSV-negative groups.¹⁰

Based on the findings in this study, it is recommended that, at the very least, urinalysis for bacterial infection be performed in all infants with RSV bronchiolitis who are younger than age 60 days. Furthermore, since there was no difference in the rates of SBI in patients younger than age 28 days, infants in this age range should undergo a full septic work-up (blood, urine, and cerebrospinal fluid)—regardless of RSV infection status. For infants between ages 28 and 60 days, there is not enough evidence to recommend for or against further laboratory evaluation other than urinalysis.

  

Treatment

Nasal Suctioning

Nasal suctioning has become the first-line treatment for infants with bronchiolitis. It is used to clear secretions from the nasal passages to aid in respiration, which is particularly important in younger infants—who are obligate nose breathers. Current recommendations are to perform suctioning with increasing respiratory effort, before feeding and before laying the infant down to sleep.¹

Bronchodilators

In the past, bronchodilators such as the β-agonist albuterol have been used to treat bronchiolitis with the idea that bronchial smooth muscle relaxation would improve clinical symptoms. In its 2006 guidelines, the AAP had recommended a trial of albuterol and continuation only if there was a documented objective response. In the 2014 updated guidelines, however, the AAP no longer recommends the use of albuterol in any capacity.

Although several meta-analyses and systematic reviews have demonstrated that bronchodilators may improve clinical symptoms scores, they did not affect disease resolution, need for hospitalization, or length of hospital stay.² In addition, a recent Cochrane systematic review noted no benefit in the clinical course of infants with bronchiolitis treated with bronchodilators, and cited the potential adverse events (tachycardia and tremors) as outweighing any potential benefit.¹¹ In addition to albuterol, the AAP no longer recommends the use of nebulized epinephrine in the treatment of bronchiolitis.²

 

Hypertonic Saline

Although hypertonic saline (HTS) has been increasingly studied for the treatment of bronchiolitis, the AAP does not recommend its use in the ED. Despite evidence that HTS may reduce hospital length of stay after 24 hours of use in settings where the typical duration of hospitalization exceeds 3 days, it has not been shown to reduce the rate of hospitalization when used in an emergency setting.²

Corticosteroids

While there is good evidence that corticosteroids are beneficial in treating some respiratory diseases, such as asthma and croup, numerous studies have repeatedly failed to show a benefit in treating bronchiolitis. One of the largest studies, a multicenter, randomized, controlled trial of dexamethasone for bronchiolitis by the Pediatric Emergency Care Applied Research Network, did not show any alteration in admission rates, respiratory status after 4 hours of observation, or length of hospital stay.¹² Accordingly, the AAP strongly recommends against the administration of corticosteroids for bronchiolitis in any setting.²

Oxygen Therapy

Oxygen therapy is often necessary in patients with bronchiolitis who demonstrate hypoxia. The definition of hypoxia in this patient population has remained variable. The AAP has established a threshold of oxyhemoglobin saturation (SpO₂) of less than 90% to define hypoxia and has empowered clinicians to not administer oxygen if the SpO₂ exceeds 90%. Based on the oxyhemoglobin dissociation curve, the authors of the AAP guidelines note that when the SpO₂ is less than 90%, small decreases in the arterial partial pressure of oxygen (PaO₂) result in large decreases in the SpO₂. When SpO₂ is greater than 90%, however, large increases in PaO₂ are associated with only small increased in SpO₂. The AAP guidelines note, “In infants and children with bronchiolitis, no data exist to suggest that such increases [in PaO₂ and SpO₂] result in any clinically significant differences in physiologic function, patient symptoms, or clinical outcomes.”²

A relatively new method of administration of oxygen to infants with bronchiolitis is via a humidified, heated, high-flow nasal cannula (HHHFNC). This therapy has been shown to generate continuous positive airway pressure, which improves respiratory effort, reduces the work of breathing, and may decrease the need for intubation.²

  

Patient Disposition

One of the most challenging tasks for emergency physicians (EPs) is determining the appropriate disposition of infants with bronchiolitis. The variable presentation and dynamic nature of the disease make this particularly difficult. Patients at high risk for apnea should be admitted to the hospital for observation and further care as needed. Admission also should be strongly considered for those with significantly increased work of breathing and tachypnea that does not improve with suctioning—especially when these interfere with feeding. Infants with poor feeding or evidence of dehydration should be admitted to the hospital for intravenous (IV) fluid hydration or nasogastric feedings. Patients with hypoxia (SpO₂ saturations <90%) should also be admitted for supplemental oxygen therapy. It should be noted, however, the AAP recommends “spot-checks” over continuous pulse oximetry in patients who do not require oxygen therapy.²

Another important factor affecting patient disposition is the ability of the caregiver to provide basic patient care and ensure close outpatient follow-up. Prior to discharge, caregivers should be educated on the highly dynamic nature of bronchiolitis and the signs and symptoms that would require prompt return to the ED—especially if the infant has risk factors for the development of severe disease.

  

Case Conclusion

Based on the patient’s symptoms, history (most notably, the recent incident of sleep apnea at home), and physical examination, the EP quickly identified this infant was at a high risk for both severe bronchiolitis and apnea and required aggressive management. Nasal suctioning was immediately performed to help clear the patient’s secretions; this, however, only slightly improved his RR and work of breathing. Although the infant’s SpO₂ was greater than 90% on room air, the EP administered oxygen via HHHFNC at 6 L per minute, which produced a significant improvement in both RR and effort.

Given the patient’s age and the presence of a fever, a urinalysis was also obtained, the results of which showed no evidence of infection. Since the patient was only able to bottle-feed for a few minutes at a time, the EP initiated IV fluid hydration and contacted the hospitalist team for inpatient admission.

The infant was gradually weaned from HHHFNC on hospital day 2 but remained with suboptimal oral intake for another 24 hours. By hospital day 4, his work of breathing had improved significantly, and he was feeding well with through the assistance of pre-feeding nasal syringe suctioning. The patient was discharged home in the care of his parents later that same day with only mild tachypnea over baseline. At discharge, the EP emphasized the importance of providing close follow-up with their son’s pediatrician. The infant continued to gradually improve as an outpatient, with resolution of nasal congestion by day 12 of his illness; he returned to his baseline breathing and feeding pattern on day 14.

 

  

---

Dr Schneider is a pediatric emergency medicine fellow, Eastern Virginia Medical School, Children’s Hospital of The King’s Daughters, Norfolk. Dr Clingenpeel is a fellowship director of pediatric emergency medicine, and an associate professor of pediatrics, Eastern Virginia Medical School, Norfolk.

Shortly after putting their son to bed, the parents of an 8-year-old boy rushed to his room after hearing loud gurgling sounds. When they entered the room, they found their son asleep but noticed he had left-sided facial twitching, was making lip-smacking movements and gurgling noises from his throat, and drooling. They called-out his name and attempted to rouse him, but the child did not respond. The movements continued for approximately 30 seconds before spontaneously resolving.

Immediately following this episode, the parents took their son to the ED for evaluation. He was afebrile with normal vital signs. The physical examination, which included a neurological examination, was normal, and both parents noted that the child appeared to be completely back to his normal behavior. The patient’s past medical and surgical histories were unremarkable. His parents stated that he had not been on any medication and denied a family history of seizures.

Overview

Seizures, the most common pediatric neurological disorder, are a frequent presentation in the ED. It is estimated that between 4% and 10% of children will have at least one seizure before age 16 years.^1,2 The highest incidence of occurrence is seen in children younger than age 3 years; this frequency decreases in older children.²

Seizures are the resulting clinical manifestations of abnormal excessive synchronized neuronal activity within the cerebral cortex. Most seizure activity is stereotypical and self-limited³ and can be divided into two main types: generalized and partial. Generalized seizures involve both cerebral hemispheres and impairment of consciousness, while partial (or focal) seizures involve a single area of one hemisphere. Generalized seizures are usually classified as convulsive or nonconvulsive and include the following subtypes: tonic-clonic, atonic, absence, and myoclonic. Partial seizures can be subdivided into simple or complex, depending on whether consciousness is impaired.

Initial Management

As with critically ill patients, the first and most vital step in managing a seizing patient is to assess the airway, breathing, and circulation. Although the airway is the most frequently compromised component, simple interventions such as jaw thrust, suctioning, or the insertion of an oral or nasopharyngeal airway are usually sufficient to maintain adequate airway. If intubation is required, use of a short-acting paralytic agent should be considered so as not to mask ongoing seizure activity.¹ Patients actively seizing at presentation should be assumed to be in status epilepticus, which is defined as seizure activity lasting longer than 5 minutes or as repetitive seizure episodes without return of consciousness between episodes.

Treatment

The benzodiazepines are the first-line treatment for status epilepticus in pediatric patients. Although intravenous (IV) lorazepam has long been the standard for seizure termination, intramuscular midazolam has been shown to be as safe and effective in patients weighing more than 13 kg in whom IV access is either not available or is difficult to obtain.⁴

Many other forms of benzodiazepines exist for seizure cessation, such as diazepam gel, which is administered rectally. Intranasal and buccal administration of midazolam also has proved effective for pediatric seizure termination.^5,6 Second-line therapies include IV fosphenytoin, levetiracetam, phenobarbital, and valproic acid.^1,7,8 In infants, phenobarbital is usually the primary second-line therapy after benzodiazepines.

History and Behavioral Observation

Given both the significant number of causes of pediatric seizures and events that can mimic childhood seizure activity, obtaining a thorough history and detailed description of the seizure episode and any preceding events are essential. It is also important to note nonseizure-related activities unique to the pediatric population (eg, Moro reflex in young infants, back-arching with gastroesophageal reflux [Sandifer syndrome], breath-holding spells, daydreaming).

Differentiating normal movements from seizures can be particularly difficult with infants. For example, repetitive bicycling movements of the legs are a common sign of seizure activity in an infant but may be mistaken as normal by parents or inexperienced observers.

West Syndrome.
West syndrome (infantile spasms) is a rare severe seizure syndrome consisting of spasmodic flexural movements of the extremities and trunk that usually presents between ages 4 to 18 months. Subtle episodes can be misinterpreted as Moro reflex, the normal sudden extension of an infant’s extremities and arching of the back occurring from birth to approximately ages 3 to 5 months.⁹

Breath-holding.
An episode of loss of consciousness with associated cyanosis, pallor, rigidity, limpness, or even twitching that was immediately preceded by vigorous crying in a child ages 6 months to 5 years should prompt the physician to strongly consider a breath-holding spell.

Attention Deficit Disorder Versus Seizure Activity.
Children, especially those with attention deficit disorder, have a propensity for daydreaming. These children will often stare and not respond to voice at times; however, if these episodes are associated with facial movements or a sudden pause in activity (also known as behavior arrest), the possibility of absence or complex partial seizures should be suspected.²

 

Physical Examination

Along with the patient’s history, the physical examination should focus primarily on determining a possible seizure etiology. The entire body should be thoroughly evaluated for evidence of trauma. The head should be carefully evaluated for signs of deformity or swelling; the fullness of the anterior fontanel should be carefully assessed; and the pupils should be examined for signs of increased intracranial pressure (ICP). In the older pediatric patient with closed fontanelles in whom a cervical spine injury has been ruled out, assessment of the neck for evidence of meningeal irritation should be performed. Stigmata of underlying medical disease, along with the presence of dysmorphic features associated with particular genetic syndromes, also should be evaluated. In addition, signs of common toxidromes should be sought.

Common Etiologies and Diagnostic Evaluation by Age

Since seizure etiologies and diagnostic evaluation vary greatly along the pediatric age spectrum, it is helpful to divide this population into three main groups: neonates and infants (ages 0-12 months), toddlers and young children (ages 12 months-10 years), and preadolescents and teenagers (ages 11-18 years).

In patients with known seizure disorders, the majority of cases are due to subtherapeutic antiepileptic medications either from a patient “outgrowing” his or her weight-based dose or from medication noncompliance. For the purposes of this article, we have limited our discussion to patients with first-time seizures.

Neonates and Infants.
In infants, hypoxic ischemic encephalopathy (HIE) due to perinatal asphyxia is the most common cause of seizures, the vast majority of which present within the first 24 to 48 hours of life.² Although EPs may encounter children with seizures caused by HIE, it is rare that these represent an initial seizure. Far more common etiologies of first-time seizures in infants are infection (eg, meningitis, encephalitis, sepsis) and nonaccidental trauma.

Electrolyte Imbalance. Although electrolytes are frequently checked in all age groups with seizures, infants are by far the most likely to experience seizures secondary to electrolyte abnormalities. Therefore, this is the only group in which routine evaluation of electrolytes is recommended. Common conditions associated with electrolyte imbalance include hyponatremia, hypocalcemia and hypomagnesemia, as well as errors of metabolism.

Hyponatremia in infants can be secondary to congenital adrenal hyperplasia or formula overdilution. Hypocalcemia and hypomagnesemia, associated with hypoparathyroidism may be the initial presentation in infants with DiGeorge syndrome. In addition, inborn errors of metabolism frequently lead to hypoglycemic seizures. Thus, in all patients with ongoing seizures refractory to medication, electrolyte abnormalities should be strongly considered.

Computed Tomography. A computed tomography (CT) scan of the head should be highly considered in this patient population as it is often difficult to determine if the patient has returned to neurological baseline. Since the fontanelles are open in infants, they can tolerate larger increases in intracranial volume (whether from blood or mass lesion) before evidence of increased ICP becomes clinically evident.

Lumbar Puncture. A lumbar puncture (LP) to analyze the cerebrospinal fluid (CSF) for infection should be considered in all afebrile infants with seizures, though some recent evidence shows a relatively low yield in such testing.^10,11

Toddlers and Young Children.
Within this age group, febrile seizures tend to predominate. A febrile seizure is defined as a seizure occurring between ages 6 months and 5 years that is associated with fever (temperature >38˚C [100.4˚F]) but without evidence of intracranial infection, neurological disease, or another defined cause.1 These seizures can be simple or complex. Simple febrile seizures last less than 15 minutes, have generalized clinical features, and occur only once in a 24-hour period. In contrast, complex febrile seizures last longer than 15 minutes, have focal manifestations, or recur multiple times in 24 hours.³

Simple Febrile Seizures.  The vast majority of patients presenting with simple febrile seizures require very limited diagnostic evaluation. If the patient has no evidence of intracranial infection, a normal neurological examination, and is back to baseline mental status, then no further evaluation is necessary. Serum electrolytes should only be checked if the patient does not quickly return to neurological baseline, at which point checking a serum glucose level would be prudent. Any further laboratory testing should be for the sole purpose of determining the source of the patient’s fever.

Most patients do not require CSF testing. In the consensus statement on the neurodiagnostic evaluation of patients with simple febrile seizures, the American Academy of Pediatrics listed only being younger than 6 months of age as a strong indicator to perform an LP. An LP should, however, be considered in patients aged 6 to 12 months who are deficient in their immunizations (especially Haemophilus influenza type B and Streptococcus pneumoniae) and in all patients pretreated with antibiotics as this could mask the signs of meningitis and encephalitis.¹²

 

Complex Febrile Seizures. Firm recommendations regarding the management of complex febrile seizures are currently lacking. These seizures carry a higher risk of intracranial infections and therefore warrant a low-threshold for both neuroimaging and CSF evaluation, especially in those patients younger than age 18 months.

Afebrile Seizures. Types of afebrile seizure disorders presenting in this age group include juvenile myoclonic epilepsy (JME) and benign rolandic epilepsy (BRE), both of which tend to present between ages 5 and 15 years. The majority of patients with first-time afebrile seizures do not require emergent neuroimaging; however, they should be referred to a pediatric neurologist for outpatient magnetic resonance imaging (MRI) of the brain and an electroencephalogram (EEG). Patients requiring emergent neuroimaging with either CT or MRI include those with signs or symptoms of elevated ICP, a focal seizure or focal findings on neurological examination, failure to return to neurological baseline, and a seizure in the setting of head trauma.¹

Juvenile Myoclonic Epilepsy. Also known as Janz syndrome, JME is one of most common types of idiopathic generalized epilepsy in childhood. It presents most commonly in otherwise healthy teenagers with one or more of the following seizure types: myoclonic jerks, generalized tonic-clonic seizures (GTCS), or absence seizures. Myoclonic jerks are unique in that they occur during the morning hours, usually the first hour after awakening. They consist of rapid muscle contractions which are most often symmetric and bilateral. The GTCS occur in about 90% of patients with JME, typically just after awakening or during sleep. Both myoclonic jerks and GTCS are exacerbated by sleep deprivation.

Absence seizures are the least common type of seizure in JME. Intelligence in these patients is normal and there is often a family history of similar seizures. Most patients respond well to treatment with antiepileptic drugs, which are usually required for life.^3,13

Benign Rolandic Epilepsy. This is the most common form of partial epilepsy in childhood. The name is derived from the central sulcus of the cerebral cortex (the rolandic fissure) around which these seizures originate. Onset of BRE typically occurs between ages 5 and 15 years, with a peak incidence of initial seizures occurring between ages 8 and 9 years. Males are more commonly affected than females (approximate distribution of 1.5:1).²

Simple partial seizures are the hallmark of this type of epilepsy, with the majority of these seizures occurring during sleep. Cardinal features include unilateral facial sensory-motor symptoms, oropharyngeal symptoms, speech arrest, and hypersalivation. Although all of these manifestations are often present, seizures may be marked by only a single symptom. Although it is uncommon, partial seizures may progress to generalized tonic-clonic activity. The hallmark finding on EEG is centrotemporal spikes. Most children do not require treatment, and the vast majority (98%) outgrow the seizures by age 18 years.³ Children with BRE have normal development and intelligence.

Early Adolescents and Teenagers.
Among this cohort, toxic ingestion and overdose tend to be the most common etiologies of first-time seizures presenting to the ED. Oral hypoglycemics (especially sulfonylureas), tricyclic antidepressants, and isoniazid are the most common prescription medications leading to seizures. Others drugs include salicylates, lithium, anticholinergic medications, and bupropion.¹ With respect to nonprescription drugs, alcohol can cause seizures via hypoglycemia; cocaine and amphetamines also have a propensity to induce seizures.¹⁴ It is paramount to evaluate serum glucose levels and consider toxicologic etiology early in the management of seizures in this age group.

Case Conclusion

Given the focal nature of this patient’s probable seizures, a CT scan of the brain was ordered without contrast to rule out an intracranial mass lesion. Based on negative findings, no further testing was ordered. The patient remained at neurological baseline throughout the course of his stay in the ED, and was discharged home with a prescription for rectal diazepam and instructions on its use for seizures lasting longer than 5 minutes. He was referred to a pediatric neurologist for further evaluation, which included an EEG study that confirmed a diagnosis of BRE.

Dr. Schneider is a pediatric emergency medicine fellow, Eastern Virginia Medical School, Children’s Hospital of the King’s Daughters, Norfolk.

Dr. Clingenpeel is a fellowship director, pediatric emergency medicine, and associate professor of pediatrics, Eastern Virginia Medical School, Norfolk.

Shortly after putting their son to bed, the parents of an 8-year-old boy rushed to his room after hearing loud gurgling sounds. When they entered the room, they found their son asleep but noticed he had left-sided facial twitching, was making lip-smacking movements and gurgling noises from his throat, and drooling. They called-out his name and attempted to rouse him, but the child did not respond. The movements continued for approximately 30 seconds before spontaneously resolving.

Immediately following this episode, the parents took their son to the ED for evaluation. He was afebrile with normal vital signs. The physical examination, which included a neurological examination, was normal, and both parents noted that the child appeared to be completely back to his normal behavior. The patient’s past medical and surgical histories were unremarkable. His parents stated that he had not been on any medication and denied a family history of seizures.

Overview

Seizures, the most common pediatric neurological disorder, are a frequent presentation in the ED. It is estimated that between 4% and 10% of children will have at least one seizure before age 16 years.^1,2 The highest incidence of occurrence is seen in children younger than age 3 years; this frequency decreases in older children.²

Seizures are the resulting clinical manifestations of abnormal excessive synchronized neuronal activity within the cerebral cortex. Most seizure activity is stereotypical and self-limited³ and can be divided into two main types: generalized and partial. Generalized seizures involve both cerebral hemispheres and impairment of consciousness, while partial (or focal) seizures involve a single area of one hemisphere. Generalized seizures are usually classified as convulsive or nonconvulsive and include the following subtypes: tonic-clonic, atonic, absence, and myoclonic. Partial seizures can be subdivided into simple or complex, depending on whether consciousness is impaired.

Initial Management

As with critically ill patients, the first and most vital step in managing a seizing patient is to assess the airway, breathing, and circulation. Although the airway is the most frequently compromised component, simple interventions such as jaw thrust, suctioning, or the insertion of an oral or nasopharyngeal airway are usually sufficient to maintain adequate airway. If intubation is required, use of a short-acting paralytic agent should be considered so as not to mask ongoing seizure activity.¹ Patients actively seizing at presentation should be assumed to be in status epilepticus, which is defined as seizure activity lasting longer than 5 minutes or as repetitive seizure episodes without return of consciousness between episodes.

Treatment

The benzodiazepines are the first-line treatment for status epilepticus in pediatric patients. Although intravenous (IV) lorazepam has long been the standard for seizure termination, intramuscular midazolam has been shown to be as safe and effective in patients weighing more than 13 kg in whom IV access is either not available or is difficult to obtain.⁴

Many other forms of benzodiazepines exist for seizure cessation, such as diazepam gel, which is administered rectally. Intranasal and buccal administration of midazolam also has proved effective for pediatric seizure termination.^5,6 Second-line therapies include IV fosphenytoin, levetiracetam, phenobarbital, and valproic acid.^1,7,8 In infants, phenobarbital is usually the primary second-line therapy after benzodiazepines.

History and Behavioral Observation

Given both the significant number of causes of pediatric seizures and events that can mimic childhood seizure activity, obtaining a thorough history and detailed description of the seizure episode and any preceding events are essential. It is also important to note nonseizure-related activities unique to the pediatric population (eg, Moro reflex in young infants, back-arching with gastroesophageal reflux [Sandifer syndrome], breath-holding spells, daydreaming).

Differentiating normal movements from seizures can be particularly difficult with infants. For example, repetitive bicycling movements of the legs are a common sign of seizure activity in an infant but may be mistaken as normal by parents or inexperienced observers.

West Syndrome.
West syndrome (infantile spasms) is a rare severe seizure syndrome consisting of spasmodic flexural movements of the extremities and trunk that usually presents between ages 4 to 18 months. Subtle episodes can be misinterpreted as Moro reflex, the normal sudden extension of an infant’s extremities and arching of the back occurring from birth to approximately ages 3 to 5 months.⁹

Breath-holding.
An episode of loss of consciousness with associated cyanosis, pallor, rigidity, limpness, or even twitching that was immediately preceded by vigorous crying in a child ages 6 months to 5 years should prompt the physician to strongly consider a breath-holding spell.

Attention Deficit Disorder Versus Seizure Activity.
Children, especially those with attention deficit disorder, have a propensity for daydreaming. These children will often stare and not respond to voice at times; however, if these episodes are associated with facial movements or a sudden pause in activity (also known as behavior arrest), the possibility of absence or complex partial seizures should be suspected.²

 

Physical Examination

Along with the patient’s history, the physical examination should focus primarily on determining a possible seizure etiology. The entire body should be thoroughly evaluated for evidence of trauma. The head should be carefully evaluated for signs of deformity or swelling; the fullness of the anterior fontanel should be carefully assessed; and the pupils should be examined for signs of increased intracranial pressure (ICP). In the older pediatric patient with closed fontanelles in whom a cervical spine injury has been ruled out, assessment of the neck for evidence of meningeal irritation should be performed. Stigmata of underlying medical disease, along with the presence of dysmorphic features associated with particular genetic syndromes, also should be evaluated. In addition, signs of common toxidromes should be sought.

Common Etiologies and Diagnostic Evaluation by Age

Since seizure etiologies and diagnostic evaluation vary greatly along the pediatric age spectrum, it is helpful to divide this population into three main groups: neonates and infants (ages 0-12 months), toddlers and young children (ages 12 months-10 years), and preadolescents and teenagers (ages 11-18 years).

In patients with known seizure disorders, the majority of cases are due to subtherapeutic antiepileptic medications either from a patient “outgrowing” his or her weight-based dose or from medication noncompliance. For the purposes of this article, we have limited our discussion to patients with first-time seizures.

Neonates and Infants.
In infants, hypoxic ischemic encephalopathy (HIE) due to perinatal asphyxia is the most common cause of seizures, the vast majority of which present within the first 24 to 48 hours of life.² Although EPs may encounter children with seizures caused by HIE, it is rare that these represent an initial seizure. Far more common etiologies of first-time seizures in infants are infection (eg, meningitis, encephalitis, sepsis) and nonaccidental trauma.

Electrolyte Imbalance. Although electrolytes are frequently checked in all age groups with seizures, infants are by far the most likely to experience seizures secondary to electrolyte abnormalities. Therefore, this is the only group in which routine evaluation of electrolytes is recommended. Common conditions associated with electrolyte imbalance include hyponatremia, hypocalcemia and hypomagnesemia, as well as errors of metabolism.

Hyponatremia in infants can be secondary to congenital adrenal hyperplasia or formula overdilution. Hypocalcemia and hypomagnesemia, associated with hypoparathyroidism may be the initial presentation in infants with DiGeorge syndrome. In addition, inborn errors of metabolism frequently lead to hypoglycemic seizures. Thus, in all patients with ongoing seizures refractory to medication, electrolyte abnormalities should be strongly considered.

Computed Tomography. A computed tomography (CT) scan of the head should be highly considered in this patient population as it is often difficult to determine if the patient has returned to neurological baseline. Since the fontanelles are open in infants, they can tolerate larger increases in intracranial volume (whether from blood or mass lesion) before evidence of increased ICP becomes clinically evident.

Lumbar Puncture. A lumbar puncture (LP) to analyze the cerebrospinal fluid (CSF) for infection should be considered in all afebrile infants with seizures, though some recent evidence shows a relatively low yield in such testing.^10,11

Toddlers and Young Children.
Within this age group, febrile seizures tend to predominate. A febrile seizure is defined as a seizure occurring between ages 6 months and 5 years that is associated with fever (temperature >38˚C [100.4˚F]) but without evidence of intracranial infection, neurological disease, or another defined cause.1 These seizures can be simple or complex. Simple febrile seizures last less than 15 minutes, have generalized clinical features, and occur only once in a 24-hour period. In contrast, complex febrile seizures last longer than 15 minutes, have focal manifestations, or recur multiple times in 24 hours.³

Simple Febrile Seizures.  The vast majority of patients presenting with simple febrile seizures require very limited diagnostic evaluation. If the patient has no evidence of intracranial infection, a normal neurological examination, and is back to baseline mental status, then no further evaluation is necessary. Serum electrolytes should only be checked if the patient does not quickly return to neurological baseline, at which point checking a serum glucose level would be prudent. Any further laboratory testing should be for the sole purpose of determining the source of the patient’s fever.

Most patients do not require CSF testing. In the consensus statement on the neurodiagnostic evaluation of patients with simple febrile seizures, the American Academy of Pediatrics listed only being younger than 6 months of age as a strong indicator to perform an LP. An LP should, however, be considered in patients aged 6 to 12 months who are deficient in their immunizations (especially Haemophilus influenza type B and Streptococcus pneumoniae) and in all patients pretreated with antibiotics as this could mask the signs of meningitis and encephalitis.¹²

 

Complex Febrile Seizures. Firm recommendations regarding the management of complex febrile seizures are currently lacking. These seizures carry a higher risk of intracranial infections and therefore warrant a low-threshold for both neuroimaging and CSF evaluation, especially in those patients younger than age 18 months.

Afebrile Seizures. Types of afebrile seizure disorders presenting in this age group include juvenile myoclonic epilepsy (JME) and benign rolandic epilepsy (BRE), both of which tend to present between ages 5 and 15 years. The majority of patients with first-time afebrile seizures do not require emergent neuroimaging; however, they should be referred to a pediatric neurologist for outpatient magnetic resonance imaging (MRI) of the brain and an electroencephalogram (EEG). Patients requiring emergent neuroimaging with either CT or MRI include those with signs or symptoms of elevated ICP, a focal seizure or focal findings on neurological examination, failure to return to neurological baseline, and a seizure in the setting of head trauma.¹

Juvenile Myoclonic Epilepsy. Also known as Janz syndrome, JME is one of most common types of idiopathic generalized epilepsy in childhood. It presents most commonly in otherwise healthy teenagers with one or more of the following seizure types: myoclonic jerks, generalized tonic-clonic seizures (GTCS), or absence seizures. Myoclonic jerks are unique in that they occur during the morning hours, usually the first hour after awakening. They consist of rapid muscle contractions which are most often symmetric and bilateral. The GTCS occur in about 90% of patients with JME, typically just after awakening or during sleep. Both myoclonic jerks and GTCS are exacerbated by sleep deprivation.

Absence seizures are the least common type of seizure in JME. Intelligence in these patients is normal and there is often a family history of similar seizures. Most patients respond well to treatment with antiepileptic drugs, which are usually required for life.^3,13

Benign Rolandic Epilepsy. This is the most common form of partial epilepsy in childhood. The name is derived from the central sulcus of the cerebral cortex (the rolandic fissure) around which these seizures originate. Onset of BRE typically occurs between ages 5 and 15 years, with a peak incidence of initial seizures occurring between ages 8 and 9 years. Males are more commonly affected than females (approximate distribution of 1.5:1).²

Simple partial seizures are the hallmark of this type of epilepsy, with the majority of these seizures occurring during sleep. Cardinal features include unilateral facial sensory-motor symptoms, oropharyngeal symptoms, speech arrest, and hypersalivation. Although all of these manifestations are often present, seizures may be marked by only a single symptom. Although it is uncommon, partial seizures may progress to generalized tonic-clonic activity. The hallmark finding on EEG is centrotemporal spikes. Most children do not require treatment, and the vast majority (98%) outgrow the seizures by age 18 years.³ Children with BRE have normal development and intelligence.

Early Adolescents and Teenagers.
Among this cohort, toxic ingestion and overdose tend to be the most common etiologies of first-time seizures presenting to the ED. Oral hypoglycemics (especially sulfonylureas), tricyclic antidepressants, and isoniazid are the most common prescription medications leading to seizures. Others drugs include salicylates, lithium, anticholinergic medications, and bupropion.¹ With respect to nonprescription drugs, alcohol can cause seizures via hypoglycemia; cocaine and amphetamines also have a propensity to induce seizures.¹⁴ It is paramount to evaluate serum glucose levels and consider toxicologic etiology early in the management of seizures in this age group.

Case Conclusion

Given the focal nature of this patient’s probable seizures, a CT scan of the brain was ordered without contrast to rule out an intracranial mass lesion. Based on negative findings, no further testing was ordered. The patient remained at neurological baseline throughout the course of his stay in the ED, and was discharged home with a prescription for rectal diazepam and instructions on its use for seizures lasting longer than 5 minutes. He was referred to a pediatric neurologist for further evaluation, which included an EEG study that confirmed a diagnosis of BRE.

Dr. Schneider is a pediatric emergency medicine fellow, Eastern Virginia Medical School, Children’s Hospital of the King’s Daughters, Norfolk.

Dr. Clingenpeel is a fellowship director, pediatric emergency medicine, and associate professor of pediatrics, Eastern Virginia Medical School, Norfolk.

Shortly after putting their son to bed, the parents of an 8-year-old boy rushed to his room after hearing loud gurgling sounds. When they entered the room, they found their son asleep but noticed he had left-sided facial twitching, was making lip-smacking movements and gurgling noises from his throat, and drooling. They called-out his name and attempted to rouse him, but the child did not respond. The movements continued for approximately 30 seconds before spontaneously resolving.

Immediately following this episode, the parents took their son to the ED for evaluation. He was afebrile with normal vital signs. The physical examination, which included a neurological examination, was normal, and both parents noted that the child appeared to be completely back to his normal behavior. The patient’s past medical and surgical histories were unremarkable. His parents stated that he had not been on any medication and denied a family history of seizures.

Overview

Seizures, the most common pediatric neurological disorder, are a frequent presentation in the ED. It is estimated that between 4% and 10% of children will have at least one seizure before age 16 years.^1,2 The highest incidence of occurrence is seen in children younger than age 3 years; this frequency decreases in older children.²

Seizures are the resulting clinical manifestations of abnormal excessive synchronized neuronal activity within the cerebral cortex. Most seizure activity is stereotypical and self-limited³ and can be divided into two main types: generalized and partial. Generalized seizures involve both cerebral hemispheres and impairment of consciousness, while partial (or focal) seizures involve a single area of one hemisphere. Generalized seizures are usually classified as convulsive or nonconvulsive and include the following subtypes: tonic-clonic, atonic, absence, and myoclonic. Partial seizures can be subdivided into simple or complex, depending on whether consciousness is impaired.

Initial Management

As with critically ill patients, the first and most vital step in managing a seizing patient is to assess the airway, breathing, and circulation. Although the airway is the most frequently compromised component, simple interventions such as jaw thrust, suctioning, or the insertion of an oral or nasopharyngeal airway are usually sufficient to maintain adequate airway. If intubation is required, use of a short-acting paralytic agent should be considered so as not to mask ongoing seizure activity.¹ Patients actively seizing at presentation should be assumed to be in status epilepticus, which is defined as seizure activity lasting longer than 5 minutes or as repetitive seizure episodes without return of consciousness between episodes.

Treatment

The benzodiazepines are the first-line treatment for status epilepticus in pediatric patients. Although intravenous (IV) lorazepam has long been the standard for seizure termination, intramuscular midazolam has been shown to be as safe and effective in patients weighing more than 13 kg in whom IV access is either not available or is difficult to obtain.⁴

Many other forms of benzodiazepines exist for seizure cessation, such as diazepam gel, which is administered rectally. Intranasal and buccal administration of midazolam also has proved effective for pediatric seizure termination.^5,6 Second-line therapies include IV fosphenytoin, levetiracetam, phenobarbital, and valproic acid.^1,7,8 In infants, phenobarbital is usually the primary second-line therapy after benzodiazepines.

History and Behavioral Observation

Given both the significant number of causes of pediatric seizures and events that can mimic childhood seizure activity, obtaining a thorough history and detailed description of the seizure episode and any preceding events are essential. It is also important to note nonseizure-related activities unique to the pediatric population (eg, Moro reflex in young infants, back-arching with gastroesophageal reflux [Sandifer syndrome], breath-holding spells, daydreaming).

Differentiating normal movements from seizures can be particularly difficult with infants. For example, repetitive bicycling movements of the legs are a common sign of seizure activity in an infant but may be mistaken as normal by parents or inexperienced observers.

West Syndrome.
West syndrome (infantile spasms) is a rare severe seizure syndrome consisting of spasmodic flexural movements of the extremities and trunk that usually presents between ages 4 to 18 months. Subtle episodes can be misinterpreted as Moro reflex, the normal sudden extension of an infant’s extremities and arching of the back occurring from birth to approximately ages 3 to 5 months.⁹

Breath-holding.
An episode of loss of consciousness with associated cyanosis, pallor, rigidity, limpness, or even twitching that was immediately preceded by vigorous crying in a child ages 6 months to 5 years should prompt the physician to strongly consider a breath-holding spell.

Attention Deficit Disorder Versus Seizure Activity.
Children, especially those with attention deficit disorder, have a propensity for daydreaming. These children will often stare and not respond to voice at times; however, if these episodes are associated with facial movements or a sudden pause in activity (also known as behavior arrest), the possibility of absence or complex partial seizures should be suspected.²

 

Physical Examination

Along with the patient’s history, the physical examination should focus primarily on determining a possible seizure etiology. The entire body should be thoroughly evaluated for evidence of trauma. The head should be carefully evaluated for signs of deformity or swelling; the fullness of the anterior fontanel should be carefully assessed; and the pupils should be examined for signs of increased intracranial pressure (ICP). In the older pediatric patient with closed fontanelles in whom a cervical spine injury has been ruled out, assessment of the neck for evidence of meningeal irritation should be performed. Stigmata of underlying medical disease, along with the presence of dysmorphic features associated with particular genetic syndromes, also should be evaluated. In addition, signs of common toxidromes should be sought.

Common Etiologies and Diagnostic Evaluation by Age

Since seizure etiologies and diagnostic evaluation vary greatly along the pediatric age spectrum, it is helpful to divide this population into three main groups: neonates and infants (ages 0-12 months), toddlers and young children (ages 12 months-10 years), and preadolescents and teenagers (ages 11-18 years).

In patients with known seizure disorders, the majority of cases are due to subtherapeutic antiepileptic medications either from a patient “outgrowing” his or her weight-based dose or from medication noncompliance. For the purposes of this article, we have limited our discussion to patients with first-time seizures.

Neonates and Infants.
In infants, hypoxic ischemic encephalopathy (HIE) due to perinatal asphyxia is the most common cause of seizures, the vast majority of which present within the first 24 to 48 hours of life.² Although EPs may encounter children with seizures caused by HIE, it is rare that these represent an initial seizure. Far more common etiologies of first-time seizures in infants are infection (eg, meningitis, encephalitis, sepsis) and nonaccidental trauma.

Electrolyte Imbalance. Although electrolytes are frequently checked in all age groups with seizures, infants are by far the most likely to experience seizures secondary to electrolyte abnormalities. Therefore, this is the only group in which routine evaluation of electrolytes is recommended. Common conditions associated with electrolyte imbalance include hyponatremia, hypocalcemia and hypomagnesemia, as well as errors of metabolism.

Hyponatremia in infants can be secondary to congenital adrenal hyperplasia or formula overdilution. Hypocalcemia and hypomagnesemia, associated with hypoparathyroidism may be the initial presentation in infants with DiGeorge syndrome. In addition, inborn errors of metabolism frequently lead to hypoglycemic seizures. Thus, in all patients with ongoing seizures refractory to medication, electrolyte abnormalities should be strongly considered.

Computed Tomography. A computed tomography (CT) scan of the head should be highly considered in this patient population as it is often difficult to determine if the patient has returned to neurological baseline. Since the fontanelles are open in infants, they can tolerate larger increases in intracranial volume (whether from blood or mass lesion) before evidence of increased ICP becomes clinically evident.

Lumbar Puncture. A lumbar puncture (LP) to analyze the cerebrospinal fluid (CSF) for infection should be considered in all afebrile infants with seizures, though some recent evidence shows a relatively low yield in such testing.^10,11

Toddlers and Young Children.
Within this age group, febrile seizures tend to predominate. A febrile seizure is defined as a seizure occurring between ages 6 months and 5 years that is associated with fever (temperature >38˚C [100.4˚F]) but without evidence of intracranial infection, neurological disease, or another defined cause.1 These seizures can be simple or complex. Simple febrile seizures last less than 15 minutes, have generalized clinical features, and occur only once in a 24-hour period. In contrast, complex febrile seizures last longer than 15 minutes, have focal manifestations, or recur multiple times in 24 hours.³

Simple Febrile Seizures.  The vast majority of patients presenting with simple febrile seizures require very limited diagnostic evaluation. If the patient has no evidence of intracranial infection, a normal neurological examination, and is back to baseline mental status, then no further evaluation is necessary. Serum electrolytes should only be checked if the patient does not quickly return to neurological baseline, at which point checking a serum glucose level would be prudent. Any further laboratory testing should be for the sole purpose of determining the source of the patient’s fever.

Most patients do not require CSF testing. In the consensus statement on the neurodiagnostic evaluation of patients with simple febrile seizures, the American Academy of Pediatrics listed only being younger than 6 months of age as a strong indicator to perform an LP. An LP should, however, be considered in patients aged 6 to 12 months who are deficient in their immunizations (especially Haemophilus influenza type B and Streptococcus pneumoniae) and in all patients pretreated with antibiotics as this could mask the signs of meningitis and encephalitis.¹²

 

Complex Febrile Seizures. Firm recommendations regarding the management of complex febrile seizures are currently lacking. These seizures carry a higher risk of intracranial infections and therefore warrant a low-threshold for both neuroimaging and CSF evaluation, especially in those patients younger than age 18 months.

Afebrile Seizures. Types of afebrile seizure disorders presenting in this age group include juvenile myoclonic epilepsy (JME) and benign rolandic epilepsy (BRE), both of which tend to present between ages 5 and 15 years. The majority of patients with first-time afebrile seizures do not require emergent neuroimaging; however, they should be referred to a pediatric neurologist for outpatient magnetic resonance imaging (MRI) of the brain and an electroencephalogram (EEG). Patients requiring emergent neuroimaging with either CT or MRI include those with signs or symptoms of elevated ICP, a focal seizure or focal findings on neurological examination, failure to return to neurological baseline, and a seizure in the setting of head trauma.¹

Juvenile Myoclonic Epilepsy. Also known as Janz syndrome, JME is one of most common types of idiopathic generalized epilepsy in childhood. It presents most commonly in otherwise healthy teenagers with one or more of the following seizure types: myoclonic jerks, generalized tonic-clonic seizures (GTCS), or absence seizures. Myoclonic jerks are unique in that they occur during the morning hours, usually the first hour after awakening. They consist of rapid muscle contractions which are most often symmetric and bilateral. The GTCS occur in about 90% of patients with JME, typically just after awakening or during sleep. Both myoclonic jerks and GTCS are exacerbated by sleep deprivation.

Absence seizures are the least common type of seizure in JME. Intelligence in these patients is normal and there is often a family history of similar seizures. Most patients respond well to treatment with antiepileptic drugs, which are usually required for life.^3,13

Benign Rolandic Epilepsy. This is the most common form of partial epilepsy in childhood. The name is derived from the central sulcus of the cerebral cortex (the rolandic fissure) around which these seizures originate. Onset of BRE typically occurs between ages 5 and 15 years, with a peak incidence of initial seizures occurring between ages 8 and 9 years. Males are more commonly affected than females (approximate distribution of 1.5:1).²

Simple partial seizures are the hallmark of this type of epilepsy, with the majority of these seizures occurring during sleep. Cardinal features include unilateral facial sensory-motor symptoms, oropharyngeal symptoms, speech arrest, and hypersalivation. Although all of these manifestations are often present, seizures may be marked by only a single symptom. Although it is uncommon, partial seizures may progress to generalized tonic-clonic activity. The hallmark finding on EEG is centrotemporal spikes. Most children do not require treatment, and the vast majority (98%) outgrow the seizures by age 18 years.³ Children with BRE have normal development and intelligence.

Early Adolescents and Teenagers.
Among this cohort, toxic ingestion and overdose tend to be the most common etiologies of first-time seizures presenting to the ED. Oral hypoglycemics (especially sulfonylureas), tricyclic antidepressants, and isoniazid are the most common prescription medications leading to seizures. Others drugs include salicylates, lithium, anticholinergic medications, and bupropion.¹ With respect to nonprescription drugs, alcohol can cause seizures via hypoglycemia; cocaine and amphetamines also have a propensity to induce seizures.¹⁴ It is paramount to evaluate serum glucose levels and consider toxicologic etiology early in the management of seizures in this age group.

Case Conclusion

Given the focal nature of this patient’s probable seizures, a CT scan of the brain was ordered without contrast to rule out an intracranial mass lesion. Based on negative findings, no further testing was ordered. The patient remained at neurological baseline throughout the course of his stay in the ED, and was discharged home with a prescription for rectal diazepam and instructions on its use for seizures lasting longer than 5 minutes. He was referred to a pediatric neurologist for further evaluation, which included an EEG study that confirmed a diagnosis of BRE.

Dr. Schneider is a pediatric emergency medicine fellow, Eastern Virginia Medical School, Children’s Hospital of the King’s Daughters, Norfolk.

Dr. Clingenpeel is a fellowship director, pediatric emergency medicine, and associate professor of pediatrics, Eastern Virginia Medical School, Norfolk.